Clinical predictors for bloodstream infection in systemic lupus erythematosus.
This study aimed to investigate the distribution of pathogenic bacteria and identify clinical predictors associated with bloodstream infection (BSI) in patients with systemic lupus erythematosus (SLE). The experimental group included 43 SLE patients with BSI (SLE-BSI), who were diagnosed and treated…
Isolated nasal septal involvement in granulomatosis with polyangiitis: Radiologic features and literature review.
Granulomatosis with polyangiitis is a necrotizing granulomatous vasculitis that predominantly involves the upper and lower respiratory tracts and the kidneys. Although sinonasal manifestations are common, they are often nonspecific and can mimic benign inflammatory conditions, leading to delayed dia…
RNA in situ hybridization for ISG15 and IFI6 highlights IFN-I activity in discoid lupus erythematosus.
IFN-I signaling is a hallmark of discoid lupus erythematosus (DLE), but routine tissue-based assays to detect this pathway are limited. Re-analysis of public transcriptomic data identified ISG15 and IFI6 as candidate IFN markers, ranking among the top differentially expressed genes in DLE datasets.…
NF‑κB signaling drives Th17‑mediated lacrimal gland injury and tear dysfunction in a murine model of primary Sjögren's syndrome.
Primary Sjögren's syndrome (pSS) is a systemic autoimmune disorder characterized by chronic inflammation of exocrine glands, resulting in lacrimal gland dysfunction, reduced tear secretion and dry eye manifestations. The nuclear factor κB (NF‑κB) signaling pathway is a centra…
Refractory severe type B insulin resistance treated with daratumumab.
Type B insulin resistance syndrome (TBIRS) is a rare autoimmune condition characterized by insulin receptor autoantibodies, often in association with systemic lupus erythematosus (SLE). It can cause extreme insulin resistance, with fewer than 200 cases described worldwide. A 24-year-old man with no…
Hereditary alpha-tryptasemia demonstrates relative basophil enrichment without signs of cellular hyperreactivity.
Hereditary alpha-tryptasemia (HαT) is an autosomal dominant trait caused by increased tryptase alpha/beta 1 (TPSAB1) copy number, resulting in elevated serum tryptase levels. Although often asymptomatic, HαT is associated with anaphylaxis, flushing, and connective tissue abnormalities. A…
Genetic contributions to systemic autoimmunity are often considered more significant in children than in adults. As such, genetic evaluation may be more frequently pursued in pediatric rheumatology patients. Motivated by the discovery of a STING-associated vasculopathy with onset in infancy (SAVI) m…
Haploinsufficiency of A20 (HA20) is a primary immune regulation disease caused by heterozygous loss-of-function variants in TNFAIP3, resulting in unchecked inflammatory signaling. HA20 is a highly heterogeneous disorder with overlapping features of autoinflammation, autoimmunity, immunodeficiency, a…